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Organomegaly, demonstrated by palpable (or documented by US, CT, or MRI):
Organomegaly, demonstrated by palpable (or documented by US, CT, or MRI):
Reference:
Verstovek S et al. Systemic mastocytosis. In: WHO Classification of Tumours Editorial Board. Haematolymphoid tumours [Internet; beta version ahead of print]. International Agency for Research on Cancer; 2023. (WHO classification of tumours series, 5th ed; vol 11). Accessed September 28, 2023. https://tumourclassification.iarc.who.int/chapters/63
ANC <1 x 109/L
Hb <10 g/dL
Platelet <100 x 109/L
Ascites and elevated liver
enzymesb with or
without
hepatomegaly or cirrhotic liver with
or without portal hypertension
Palpable splenomegaly with hypersplenism with or without weight loss, with or without hypoalbuminemia
Malabsorption with hypoalbuminemia with or without weight loss
Large-sized osteolysis (≥20 mm) with or without pathologic fracture with or without bone pain
References:
1. Verstovek S et al. Systemic mastocytosis. In: WHO Classification of Tumours Editorial Board. Haematolymphoid tumours [Internet; beta version ahead of print]. International Agency for Research on Cancer; 2023. (WHO classification of tumours series, 5th ed; vol 11). Accessed September 15, 2023. https://tumourclassification.iarc.who.int/chapters/63 2. Pardanani A. Am J Hematol. 2023;98(7):1097-1116.
Maculopapular cutaneous lesions on torso since childhood1,2
Anaphylaxis due to bee venom allergies1,3
Modified and restricted diet with cromolyn has improved control of diarrhea4
Failed symptomatic treatment with montelukast, omalizumab, and prednisone1
References:
1. Pardanani A. Am J Hematol. 2023;98(7):1097-1116. 2. Hartmann K et al. J Allergy Clin Immunol. 2016;137(1):35-45. 3. Theoharides TC et al. N Engl J Med. 2015;373(2):163-172. 4. Ramsay DB et al. Gastroenterol Hepatol (N Y). 2010;6(12):772-777.
Cimetidine1
Cromolyn2
Diphenhydramine PRN3
Doxepin4
Epinephrine autoinjector at home PRN1
Hydroxyzine PRN1
PRN, as needed.
References:
1. Pardanani A. Am J Hematol. 2023;98(7):1097-1116.
2. Ramsay DB et al. Gastroenterol Hepatol (N Y). 2010;6(12):772-777.
3. Buonomo A et al. Mediterr J Hematol Infect Dis. 2022;14(1):e2022040.
4. Cardet JC et al. Expert Opin Pharmacother. 2013;14(15):2033-2045.
Peripheral blood test: CBC, chemistry profile, and liver function tests are normal; overall stable organ function1
Follow-up peripheral blood test: confirmed presence of CD25-positive MCs1,2
Serum tryptase: 28 ng/mL2,3
Follow-up tryptase: persistently elevated at 31 ng/mL2,3
KIT D816V mutation status: positive1,2
Follow-up evaluation: negative for B- and C-findings1,2
Bone marrow biopsy: confirmed the presence of multifocal dense infiltrates of
MCs
(≥15 MCs in aggregates)1,2
CBC, complete blood count; MC, mast cell.
References:
1. Pardanani A. Am J Hematol. 2023;98(7):1097-1116. 2. Verstovek S, Colmenero I, Cozzolino I, et al. Systemic mastocytosis. In: WHO Classification of Tumours Editorial Board. Haematolymphoid tumours [Internet; beta version ahead of print]. International Agency for Research on Cancer; 2023. (WHO classification of tumours series, 5th ed; vol 11). Accessed September 28, 2023. https://tumourclassification.iarc.who.int/chapters/63. 3. Akin C et al. Immunol Allergy Clin North Am. 2014;34:207-218.
(1 major + ≥1 minor or ≥3 minor)
Multifocal dense infiltrates of MCs (≥15 MCs in aggregates) are detected in
sections of bone marrow
and/or other extracutaneous organ(s)
Activating KIT point mutation(s) at codon 816 or in other critical regions of KIT in bone marrow or other extracutaneous organ(s)
Baseline serum tryptase concentration >20 ng/mL in the absence of a
myeloid-associated hematologic neoplasmb
Peripheral blood test may be used to screen for SM1-3
>25% of all MCs are atypical cells (type I or type II) on bone marrow smears
or are spindle-shaped in dense
and diffuse MC infiltrates in
sections of bone marrow or other extracutaneous organ(s)
MCs in bone marrow, blood, or another extracutaneous organ(s) aberrantly express 1 or
more of the
following antigens: CD2, CD25, CD30
Diagnosis:
1 major + 3 minor criteria with 0 B-findings suggest that Tim may have ISM, according to the WHO
Diagnostic Guidelines for SM.1,a
a Please note, these are the anticipated changes to the WHO guidelines, 5th
edition, volume 11; cited from the beta version (ahead of print) available
online.1
b Patients with ISM do not always have serum tryptase >20 ng/mL. Tryptase >20 ng/mL is considered a
minor diagnostic criterion, but basal serum tryptase levels <20 ng/mL might not rule out
SM.1,4
References:
1. Verstovek S et al. Systemic mastocytosis. In: WHO Classification of Tumours Editorial Board. Haematolymphoid tumours [Internet; beta version ahead of print]. International Agency for Research on Cancer; 2023. (WHO classification of tumours series, 5th ed; vol 11). Accessed September 28, 2023. https://tumourclassification.iarc.who.int/chapters/63 2. Hoermann G et al. J Allergy Clin Immunol Pract. 2022;10(8):1953-1963. 3. Matito A et al. PLoS One. 2013;8(10):e76116. 4. Akin C, Valent P. Immunol Allergy Clin North Am. 2014;34(2):207-218.
Peripheral blood test: CBC and chemistry profile are normal1
Serum tryptase: elevated, 48 ng/mL1,2
Follow-up tryptase: persistently elevated at 35 ng/mL1,2
KIT D816V mutation status: positive2,3
TPSAB1 gene status: total number of TPSAB1 and TPSAB2 = 4 (normal);
hereditary alpha tryptasemia ruled out4
Bone marrow biopsy: revealed multifocal dense MC infiltrates of
≥15 MC in aggregates1,2
Follow-up evaluation: negative for B- and C-findings1,2
CBC, complete blood count; MC, mast cell.
References:
1. Pardanani A. Am J Hematol. 2023;98(7):1097-1116. 2. Verstovek S et al. Systemic mastocytosis. In: WHO Classification of Tumours Editorial Board. Haematolymphoid tumours [Internet; beta version ahead of print]. International Agency for Research on Cancer; 2023. (WHO classification of tumours series, 5th ed; vol 11). Accessed September 28, 2023. https://tumourclassification.iarc.who.int/chapters/63 3.Valent P et al. Int J Mol Sci. 2019;20:2976. 4. Lyons JJ. Immunol Allergy Clin North Am. 2018;38(3):483-495.
(1 major + ≥1 minor or ≥3 minor)
Multifocal dense infiltrates of MCs (≥15 MCs in aggregates) are detected in
sections of bone marrow
and/or other extracutaneous organ(s)
Activating KIT point mutation(s) at codon 816 or in other critical regions of KIT in bone marrow or other extracutaneous organ(s)
Baseline serum tryptase concentration >20 ng/mL in the absence of a
myeloid-associated hematologic neoplasmb
Peripheral blood test may be used to screen for SM1-3
>25% of all MCs are atypical cells (type I or type II) on bone marrow smears
or are spindle-shaped in dense
and diffuse MC infiltrates in
sections of bone marrow or other extracutaneous organ(s)
MCs in bone marrow, blood, or another extracutaneous organ(s) aberrantly express 1 or
more of the
following antigens: CD2, CD25, CD30
Diagnosis: 1 major + 2 minor criteria with 0 B- or C-findings suggest that Amy may have ISM, according to the WHO Diagnostic Guidelines for SM.1,a
a Please note, these are the anticipated changes to the WHO guidelines, 5th
edition, volume 11; cited from the beta version (ahead of print) available
online.1
b Patients with ISM do not always have serum tryptase >20 ng/mL. Tryptase >20 ng/mL is considered a
minor diagnostic criterion, but basal serum tryptase levels <20 ng/mL might not rule out
SM.1,4
References:
1. Verstovek S et al. Systemic mastocytosis. In: WHO Classification of Tumours Editorial Board. Haematolymphoid tumours [Internet; beta version ahead of print]. International Agency for Research on Cancer; 2023. (WHO classification of tumours series, 5th ed; vol 11). Accessed September 28, 2023. https://tumourclassification.iarc.who.int/chapters/63 2. Hoermann G et al. J Allergy Clin Immunol Pract. 2022;10(8):1953-1963. 3. Matito A et al. PLoS One. 2013;8(10):e76116. 4. Akin C, Valent P. Immunol Allergy Clin North Am. 2014;34(2):207-218.
Peripheral blood test: CBC and chemistry profile are normal1
Serum tryptase: 9 ng/mL1,2
Follow-up tryptase: 10 ng/mL1,2
KIT D816V mutation status: negative1,2
GI biopsy: did not confirm presence of CD2-, CD25-, or
CD30-positive MCs2
Follow-up evaluation: negative for B- and C-findings2
CBC, complete blood count; MC, mast cell.
References:
1. Pardanani A. Am J Hematol. 2023;98(7):1097-1116.
2. Verstovek S et al. Systemic mastocytosis. In: WHO Classification of Tumours Editorial
Board. Haematolymphoid tumours [Internet; beta version
ahead of print].
International Agency for Research on Cancer; 2023. (WHO classification of tumours series, 5th ed; vol 11).
Accessed September 28, 2023.
https://tumourclassification.iarc.who.int/chapters/63
(1 major + ≥1 minor or ≥3 minor)
Multifocal dense infiltrates of MCs (≥15 MCs in aggregates) are detected in
sections of bone marrow
and/or other extracutaneous organ(s)
Activating KIT point mutation(s) at codon 816 or in other critical regions of KIT in bone marrow or other extracutaneous organ(s)
Baseline serum tryptase concentration >20 ng/mL in the absence of a
myeloid-associated hematologic neoplasmb
Peripheral blood test may be used
to screen for SM1-3
>25% of all MCs are atypical cells (type I or type II) on bone marrow smears
or are spindle-shaped in dense
and diffuse MC infiltrates in
sections of bone marrow or other extracutaneous organ(s)
MCs in bone marrow, blood, or another extracutaneous organ(s) aberrantly express 1 or
more of the
following antigens: CD2, CD25, CD30
Diagnosis:
Consider other differentials for Mary. 0 major and/or minor criteria
with 0 B- or C-findings suggest that Mary may not have ISM, according to
the WHO Diagnostic Guidelines for SM.1,a
a Please note, these are the anticipated changes to the WHO guidelines, 5th
edition, volume 11; cited from the beta version (ahead of print) available
online.1
b Patients with ISM do not always have serum tryptase >20 ng/mL. Tryptase >20 ng/mL is considered a
minor diagnostic criterion, but basal serum tryptase levels <20 ng/mL might not rule out
SM.1,4
References:
1. Verstovek S et al. Systemic mastocytosis. In: WHO Classification
of Tumours Editorial Board. Haematolymphoid tumours [Internet; beta version ahead of print]. International
Agency for Research on
Cancer; 2023. (WHO classification of tumours series, 5th
ed; vol 11). Accessed September 28, 2023. https://tumourclassification.iarc.who.int/chapters/63
2. Hoermann G et al. J Allergy Clin Immunol Pract. 2022;10(8):1953-1963.
3. Matito A et al. PLoS One. 2013;8(10):e76116. 4. Akin C, Valent
P. Immunol Allergy Clin North Am. 2014;34(2):207-218.
The information presented here outlines general characteristics of each disorder. Presentation of signs and symptoms may vary from patient to patient.
Cutaneous mastocytosis (CM) is a form of mastocytosis that affects the skin, with no evidence of systemic mast cell involvement, in primarily pediatric patients.1-3
Please note: Most patients diagnosed with CM are children, whereas SM is usually diagnosed in
adults.2-4 Adults with skin lesions
may receive a provisional
diagnosis of mastocytosis in the skin prior to undergoing complete staging, including bone marrow
analysis, to confirm/exclude CM or SM.4,5
ISM | CM |
---|---|
DERMATOLOGIC | |
Darier sign: Elicited upon stroking of lesioned skin1,2 Darier sign: Elicited upon stroking of lesioned skin1,2 a Please note, the Darier sign depicted here indicates a
wheal-and-flare reaction, which was developed upon stroking of the lesion in a patient with
CM. The Darier sign is an important clinical feature of mastocytosis skin lesions in
patients with CM and SM.1,2 |
|
Monomorphic maculopapular lesions, round, brown or red in color arise
predominantly on the thigh and trunk. Lesions may spread or spontaneously disappear, which
may or may not Symptoms such as flushing, pruritus, and positive Darier sign may be observed1,7,8 Patient permissions and image reprint from Mikkelsen et al. Dermatol Reports. 2014;6(1):5199.doi:10.4081/dr.2014.5199 have been granted under the Creative Commons License 4.0 https://creativecommons.org/licenses/by-nc/4.0/legalcode |
Maculopapular CM, previously known as urticaria pigmentosa, is typically characterized by round brown or red skin lesions1,2 Polymorphic, larger lesions, may present Monomorphic, small round lesions may be Patient permissions and image reprint from Hartmann K et al. |
Not usually observed1,2 |
Diffuse CM (DCM), characterized by Patient permissions and image reprint from Hartmann K et al. |
Not usually observed1,2 |
Mastocytoma, typically presents as 1-3 These lesions or blisters can be isolated or multilocalized9 Patient permissions and image reprint from Hartmann K et al. |
GASTROINTESTINAL | |
GI involvement is commonly reported in patients with ISM7,10,11 Symptoms may include diarrhea, abdominal cramping, bloating, |
Not usually observed1-3 |
SYSTEMIC | |
Systemic symptoms include fatigue, anaphylaxis, and weight loss7,11-13 Anaphylaxis may occur; patients typically present with hypotensive Episodes of anaphylaxis appear more likely to develop in patients with mastocytosis
compared to the general population; Hymenoptera |
Anaphylaxis is uncommon but may be possible1,2,17 |
OTHER | |
Additional symptoms such as cognitive impairment, dizziness, Please note that this list is not inclusive of all symptoms patients with |
Involvement is most commonly seen in the skin1,2 |
This section provides some information that may be helpful in further identifying patients with ISM vs CM and is not inclusive of all distinguishing features. Please refer to the formal guidelines for each disorder, if available, for a more comprehensive list.
In pediatric patients, the typical course of CM is usually temporary and often resolves spontaneously
around puberty, whereas
ISM in adults is usually chronic.1,2
GI, gastrointestinal; ISM, indolent systemic mastocytosis; SM, systemic mastocytosis.
References:
1. Hartmann K et al. J Allergy Clin Immunol. 2016;137(1):35-45.
2. Manjaly Thomas ZR, Hartmann, K. In: Akin C, ed. Mastocytosis. Springer,
Cham; 2020:69-91. 3. Akin C et al. In: Akin C, ed. Mastocytosis. Springer,
Cham; 2020:23-34. 4. Valent P et al. Hemasphere. 2021;5(11):e646.
5. Valent P et al. Allergy. 2014;69(10):1267-1274. 6.
Mikkelsen CS et al. Dermatol Reports. 2014;6(1):5199. 7. Pyatilova P et al.
J Allergy Clin Immunol Pract. 2022;10(8):2015-2024. 8. Hermans MAW et al.
Eur J Intern Med. 2016;30:25-30. 9. Verstovek S et al. Systemic mastocytosis.
In: WHO Classification of Tumours Editorial Board. Haematolymphoid tumours [Internet; beta version ahead
of print]. International Agency for Research on Cancer; 2023. (WHO classification of tumours series, 5th
ed; vol 11). Accessed September 28, 2023. https://tumourclassification.iarc.who.int/chapters/63
10. Hamilton MJ. In: Akin E, ed. Mastocytosis.
Springer, Cham; 2020:115-122. 11. Lim KH et al. Blood. 2009;113(23):5727-5736.
12. Taylor F et al. Orphanet J Rare Dis. 2021;16(1):414. 13.
Gülen T et al. In: Akin C, ed. Mastocytosis. Springer, Cham; 2020:141-155. 14.
Alvarez-Twose I et al. J Allergy Clin Immunol. 2010;125(6):1269-1278.e2. 15.
Pieri L et al. Am J Hematol. 2016;91(7):692-699. 16. González de Olano D et
al. J Allergy Clin Immunol. 2008;121(2):519-526. 17. Brockow K et al.
Allergy. 2008;63(2):226-232. 18. Pardanani A et al. Leukemia.
2013;27(10):2091-2094.
The information presented here outlines general characteristics of each disorder. Presentation of signs and symptoms may vary from patient to patient.
Chronic spontaneous urticaria (CSU) is a spontaneous appearance of urticaria, defined
as wheals (hives), angioedema, or both for >6 weeks
due to known or unknown
causes.1,2
ISM | CSU |
---|---|
DERMATOLOGIC | |
Monomorphic maculopapular lesions, round, brown or red in color arise
predominantly on the thigh and trunk. Symptoms such as flushing, pruritus, and positive Darier sign may be observed4,6,7 The Darier sign is an important clinical feature of mastocytosis skin
lesions. It is defined by the development of a Patient permissions and image reprint from Mikkelsen et al. Dermatol Reports. 2014;6(1):5199. doi:10.4081/dr.2014.5199 have been granted under the Creative Commons License 4.0 https://creativecommons.org/licenses/by-nc/4.0/legalcode |
Wheals are defined by swelling and Angioedema is characterized by sudden Patient permissions and image reprint from Sussman G et al. Allergy Asthma Clin Immunol. 2015;11(1):7. doi: 10.1186/s13223-015-0072-2 have been granted under the Creative Commons License 4.0 https://creativecommons.org/licenses/by-nc/4.0/legalcode |
GASTROINTESTINAL | |
GI involvement is commonly reported in patients with ISM6,9,10 Symptoms may include diarrhea, abdominal cramping, bloating, |
Some patients can have GI symptoms such as nausea, vomiting, |
SYSTEMIC | |
Systemic symptoms include fatigue, anaphylaxis, and weight loss6,10,11,13 Anaphylaxis may occur; patients typically present with hypotensive syncope and without flushing, urticaria, pruritus, and angioedema6,14,15 Episodes of anaphylaxis appear more likely to develop in patients with mastocytosis compared to the general population; Hymenoptera stings are a common trigger6,16,17 |
Not usually observed1,2 |
OTHER | |
Additional symptoms such as cognitive impairment, dizziness, Please note that this list is not inclusive of all symptoms patients with |
Some additional manifestations such as Kounis syndrome, hypertension, striatum dysfunction, respiratory symptoms, arthritis, arthralgia, or osteoporosis may occur12 |
This section provides some information that may be helpful in further identifying patients with ISM vs CSU and is not inclusive of all distinguishing features. Please refer to the formal guidelines for each disorder, if available, for a more comprehensive list.
CSU may occur with daily/almost daily signs and symptoms or as an intermittent/recurrent course. CSU
may also recur after
months or years of full remission. ISM is usually
chronic.1,19
GI, gastrointestinal; ISM, indolent systemic mastocytosis.
References:
1. Zuberbier T et al. Allergy. 2022;77(3):734-766. 2. Vestergaard C et al. Ther Adv Chronic Dis. 2015;6(6):304-313. 3. Mikkelsen CS et al. Dermatol Reports. 2014;6(1):5199. 4. Hartmann K et al. J Allergy Clin Immunol. 2016;137(1):35-45. 5. Manjaly Thomas ZR, Hartmann, K. In: Akin C, ed. Mastocytosis. Springer, Cham; 2020:69-91. 6. Pyatilova P et al. J Allergy Clin Immunol Pract. 2022;10(8):2015-2024. 7. Hermans MAW et al. Eur J Intern Med. 2016;30:25-30. 8. Sussman G et al. Allergy Asthma Clin Immunol. 2015;11(1):7. 9. Hamilton MJ. In: Akin E, ed. Mastocytosis. Springer, Cham; 2020:115-122. 10. Lim KH et al. Blood. 2009;113(23): 5727-5736. 11. Taylor F et al. Orphanet J Rare Dis. 2021;16(1):414. 12. Kocaturk E et al. Clin Transl Allergy. 2019;9:48. 13. Gülen T et al. In: Akin C, ed. Mastocytosis. Springer, Cham; 2020:141-155. 14. Verstovek S et al. Systemic mastocytosis. In: WHO Classification of Tumours Editorial Board. Haematolymphoid tumours [Internet; beta version ahead of print]. International Agency for Research on Cancer; 2023. (WHO classification of tumours series, 5th ed; vol 11). Accessed September 28, 2023. https://tumourclassification.iarc.who.int/chapters/63 15. Alvarez-Twose I et al. J Allergy Clin Immunol. 2010;125(6):1269-1278.e2. 16. Pieri L et al. Am J Hematol. 2016;91(7):692-699. 17. González de Olano D et al. J Allergy Clin Immunol. 2008;121(2):519-526. 18. Pardanani A et al. Leukemia. 2013;27(10):2091-2094. 19. Bibi S et al. In: Akin C, ed. Mastocytosis. Springer, Cham; 2020:207-230.
The information presented here outlines general characteristics of each disorder. Presentation of signs and symptoms may vary from patient to patient.
Mast cell activation syndromes (MCAS) are a clustering of disorders characterized by
the accumulation of MCs in tissues and organs,
and/or release of MC
mediators, with symptoms related to MC degranulation and mediator release.1 MCAS can be divided
into primary,
secondary, and idiopathic.2,3 Primary MCAS or
monoclonal MCAS (mMCAS) is characterized by lack of cutaneous findings and presence of
KIT D816V mutation or expression of CD25 in MCs.3
ISM | MCAS/mMCAS |
---|---|
DERMATOLOGIC | |
Monomorphic maculopapular lesions, round, brown or red in color arise predominantly on the thigh and trunk. Lesions may spread or spontaneously disappear, which may or may not indicate disease progression5-7 Symptoms such as flushing, pruritus, and positive Darier sign may be observed5,7,8 The Darier sign is an important clinical feature of mastocytosis skin
lesions. It is defined by the development of a
Patient permissions and image reprint from Mikkelsen et al. Dermatol Reports. 2014;6(1):5199. doi:10.4081/dr.2014.5199 have been granted under the Creative Commons License 4.0 https://creativecommons.org/licenses/by-nc/4.0/legalcode |
Episodic symptoms such as pruritus, urticaria, and flushing |
GASTROINTESTINAL | |
GI involvement is commonly reported in patients with ISM7,11,12 Symptoms may include diarrhea, abdominal cramping, bloating, |
Patients may present with nausea, vomiting, cramping |
SYSTEMIC | |
Systemic symptoms include fatigue, anaphylaxis, and weight loss7,12-14 Anaphylaxis may occur; patients typically present with hypotensive syncope and without flushing, urticaria, pruritus, and angioedema7,15,16 Episodes of anaphylaxis appear more likely to develop in patients with mastocytosis compared to the general population; Hymenoptera stings are a common trigger7,17,18 |
During episodes of anaphylaxis, cardiovascular, respiratory, |
OTHER | |
Additional symptoms such as cognitive impairment, dizziness, Please note that this list is not inclusive of all symptoms patients |
Chronic urticaria and angioedema can be a feature of secondary and idiopathic MCAS3 Additional symptoms such as headache, tachycardia, and fatigue |
This section provides some information that may be helpful in further identifying patients with ISM vs MCAS and is not inclusive of all distinguishing features. Please refer to the formal guidelines for each disorder, if available, for a more comprehensive list.
Basal serum tryptase levels in patients with MCAS/mMCAS are usually normal or mildly increased. If
patients with suspected MC disorders
have elevated tryptase levels,
evaluation of systemic mastocytosis or hereditary alpha tryptasemia may be warranted.2,10,20
GI, gastrointestinal; ISM, indolent systemic mastocytosis; MC, mast cell.
References:
1. Jackson CW et al. Int J Mol Sci. 2021;22(20):11270. 2. Akin C et al. J Allergy Clin Immunol. 2010;126(6):1099-1104.e4. 3. Akin C. J Allergy Clin Immunol. 2017;140(2):349-355. 4. Mikkelsen CS et al. Dermatol Reports. 2014;6(1):5199. 5. Hartmann K et al. J Allergy Clin Immunol. 2016;137(1):35-45. 6. Manjaly Thomas ZR, Hartmann, K. In: Akin C, ed. Mastocytosis. Springer, Cham; 2020:69-91. 7. Pyatilova P et al. J Allergy Clin Immunol Pract. 2022;10(8):2015-2024. 8. Hermans MAW et al. Eur J Intern Med. 2016;30:25-30. 9. Rossignol J et al. F1000Res. 2019;8:F1000 Faculty Rev-1961. 10. Weiler CR et al. J Allergy Clin Immunol. 2019;144:883. 11. Hamilton MJ. In: Akin E, ed. Mastocytosis. Springer, Cham; 2020:115-122. 12. Lim KH et al. Blood. 2009;113(23):5727-5736. 13. Taylor F et al. Orphanet J Rare Dis. 2021;16(1):414. 14. Gülen T et al. In: Akin C, ed. Mastocytosis. Springer, Cham; 2020:141-155. 15. Verstovek S et al. Systemic mastocytosis. In: WHO Classification of Tumours Editorial Board. Haematolymphoid tumours [Internet; beta version ahead of print]. International Agency for Research on Cancer; 2023. (WHO classification of tumours series, 5th ed; vol 11). Accessed September 28, 2023. https://tumourclassification.iarc.who.int/chapters/63 16. Alvarez-Twose I et al. J Allergy Clin Immunol. 2010;125(6):1269-1278.e2. 17. Pieri L et al. Am J Hematol. 2016;91(7):692-699. 18. González de Olano D et al. J Allergy Clin Immunol. 2008;121(2):519-526. 19. Pardanani A et al. Leukemia. 2013;27(10):2091-2094. 20. American Academy of Allergy, Asthma, and Immunology and American College of Allergy, Asthma, and Immunology. Anaphylaxis: a 2023 practice parameter update, 2023. Accessed September 28, 2023. https://college.acaai.org/wp-content/uploads/2023/04/Anaphylaxis-2023-Practice-Parameter-Update-01APR2023.pdf
The information presented here outlines general characteristics of each disorder. Presentation of signs and symptoms may vary from patient to patient.
Hereditary alpha tryptasemia (HαT) is a disorder characterized by an autosomal-dominant
genetic trait with extra copies of the
TPSAB1 gene, which encodes
α-tryptase.1-3 HαT is associated with elevated baseline serum tryptase levels (>8
ng/mL).1-3
ISM | HαT |
---|---|
DERMATOLOGIC | |
Monomorphic maculopapular lesions, round, brown or red in color arise predominantly on the thigh and trunk. Lesions may spread or spontaneously disappear, which may or may not indicate disease progression5-7 Symptoms such as flushing, pruritus, and positive Darier sign may be observed5,7,8 The Darier sign is an important clinical feature of mastocytosis skin
lesions. It is defined by the development of a Patient permissions and image reprint from Mikkelsen et al. Dermatol Reports. 2014;6(1):5199. doi:10.4081/dr.2014.5199 have been granted under the Creative Commons License 4.0 https://creativecommons.org/licenses/by-nc/4.0/legalcode |
Typically characterized by recurrent cutaneous symptoms, Angioedema and urticaria are commonly reported9,10 |
GASTROINTESTINAL | |
GI involvement is commonly reported in patients with ISM7,11,12 Symptoms may include diarrhea, abdominal cramping, bloating, |
IBS-like symptoms or symptoms of chronic gastroesophageal reflux are commonly present1,3 |
SYSTEMIC | |
Systemic symptoms include fatigue, anaphylaxis, and weight loss7,12-14 Anaphylaxis may occur; patients typically present with hypotensive syncope and without flushing, urticaria, pruritus, and angioedema7,15,16 Episodes of anaphylaxis appear more likely to develop in patients |
Systemic reaction consistent with IgE-mediated hypersensitivity |
OTHER | |
Additional symptoms such as cognitive impairment, dizziness, Please note that this list is not inclusive of all symptoms patients with |
Dysautonomia, arthralgia, primary dentition, headache, body pain, |
This section provides some information that may be helpful in further identifying patients with ISM vs HαT and is not inclusive of all distinguishing features. Please refer to the formal guidelines for each disorder, if available, for a more comprehensive list.
Patients with SM may simultaneously have HαT, especially among patients with ISM and BMM. In patients
with mastocytosis, presence of
HαT can indicate increased risk and
severity for anaphylaxis.7,21-23
BMM, bone marrow mastocytosis; GI, gastrointestinal; IBS, irritable bowel syndrome; IgE, immunoglobulin E; ISM, indolent systemic mastocytosis; MC, mast cell; SM, systemic mastocytosis.
References:
1. Gotlib J et al. J Allergy Clin Immunol. 2021;147(6):2043-2052. 2. Lyons JJ et al. Nat Genet. 2016;48(12):1564-1569. 3. Lyons JJ. Immunol Allergy Clin North Am. 2018;38(3):483-495. 4. Mikkelsen CS et al. Dermatol Reports. 2014;6(1):5199. 5. Hartmann K et al. J Allergy Clin Immunol. 2016;137(1):35-45. 6. Manjaly Thomas ZR, Hartmann, K. In: Akin C, ed. Mastocytosis. Springer, Cham; 2020:69-91. 7. Pyatilova P et al. J Allergy Clin Immunol Pract. 2022;10(8):2015-2024. 8. Hermans MAW et al. Eur J Intern Med. 2016;30:25-30. 9. Lyons JJ, Shwartz, LB. In: Akin C, ed. Mastocytosis. Springer, Cham; 2020:35-54. 10. Chollet MB, Akin C. J Allergy Clin Immunol. 2022;149(2):728-735.e2. 11. Hamilton MJ. In: Akin C, ed. Mastocytosis. Springer, Cham. 2020:115-122. 12. Lim KH et al. Blood. 2009;113(23):5727-5736. 13. Taylor F et al. Orphanet J Rare Dis. 2021;16(1):414. 14. Gülen T et al. In: Akin C, ed. Mastocytosis. Springer, Cham; 2020:141-155. 15. Verstovek S et al. Systemic mastocytosis. In: WHO Classification of Tumours Editorial Board. Haematolymphoid tumours [Internet; beta version ahead of print]. International Agency for Research on Cancer; 2023. (WHO classification of tumours series, 5th ed; vol 11). Accessed September 28, 2023. https://tumourclassification.iarc.who.int/chapters/63 16. Alvarez-Twose I et al. J Allergy Clin Immunol. 2010;125(6):1269-1278.e2. 17. Pieri L et al. Am J Hematol. 2016;91(7):692-699. 18. González de Olano D et al. J Allergy Clin Immunol. 2008;121(2):519-526. 19. Jackson CW et al. Int J Mol Sci. 2021;22(20):11270. 20. Pardanani A et al. Leukemia. 2013;27(10):2091-2094 21. Lyons JJ et al. J Allergy Clin Immunol. 2021;147(2):622-632. 22. American Academy of Allergy, Asthma, and Immunology and American College of Allergy, Asthma, and Immunology. Anaphylaxis: a 2023 practice parameter update, 2023. Accessed September 29, 2023. https://college.acaai.org/wp-content/uploads/2023/04/Anaphylaxis-2023-Practice-Parameter-Update-01APR2023.pdf. 23. Sordi B et al. J Allergy Clin Immunol. 2023;151(2):485-493.e11.
The information presented here outlines general characteristics of each disorder. Presentation of signs and symptoms may vary from patient to patient.
Hypereosinophilic syndrome (HES) is a heterogeneous group of rare disorders
characterized by persistently elevated eosinophil count
and
eosinophil-mediated organ damage.1,2
ISM | HES |
---|---|
DERMATOLOGIC | |
Monomorphic maculopapular lesions, round, brown or red in color arise predominantly on the thigh and trunk. Lesions may spread or spontaneously disappear, which may or may not indicate disease progression4-6 Symptoms such as flushing, pruritus, and positive Darier sign may be observed4,6,7 The Darier sign is an important clinical feature of mastocytosis skin
lesions. It is defined by the development of a Patient permissions and image reprint from Mikkelsen et al. Dermatol Reports. 2014;6(1):5199. doi:10.4081/dr.2014.5199 have been granted under the Creative Commons License 4.0 https://creativecommons.org/licenses/by-nc/4.0/legalcode |
Cutaneous manifestations such as urticaria, erythema, |
GASTROINTESTINAL | |
GI involvement is commonly reported in patients with ISM6,8,9 Symptoms may include diarrhea, abdominal cramping, |
Abdominal pain, diarrhea, nausea, and vomiting1,2 Eosinophilic gastritis, enterocolitis, or colitis may be present2 |
SYSTEMIC | |
Systemic symptoms include fatigue, anaphylaxis, and weight loss6,9-11 Anaphylaxis may occur; patients typically present with hypotensive syncope and without
flushing, urticaria, pruritus, and Episodes of anaphylaxis appear more likely to develop in patients with mastocytosis
compared to the general population; Hymenoptera |
Weakness, fatigue, and fever may occur1,2,16 |
OTHER | |
Additional symptoms such as cognitive impairment, dizziness, Please note that this list is not inclusive of all symptoms patients with |
Cough, shortness of breath, myalgias, and other cardiac, neurological |
GI, gastrointestinal; ISM, indolent systemic mastocytosis.
References:
1. Schuster B et al. Allergy. 2020;75(11):3003-3006.
2. Roufosse FE et al. Orphanet J Rare Dis. 2007;2:37. 3.
Mikkelsen CS et al. Dermatol Reports. 2014;6(1):5199. 4. Hartmann K et al.
J Allergy Clin Immunol. 2016;137(1):35-45. 5. Manjaly Thomas ZR, Hartmann K.
In: Akin C, ed. Mastocytosis. Springer, Cham; 2020:69-91. 6. Pyatilova P et
al. J Allergy Clin Immunol Pract. 2022;10(8):2015-2024. 7. Hermans MAW et al.
Eur J Intern Med. 2016;30:25-30. 8. Hamilton MJ. In: Akin E, ed.
Mastocytosis. Springer, Cham; 2020:115-122. 9. Lim KH et al. Blood.
2009;113(23):5727-5736. 10. Taylor F et al. Orphanet J Rare Dis.
2021;16(1):414. 11. Gülen
T et al. In: Akin C, ed.
Mastocytosis. Springer, Cham; 2020:141-155. 12. Verstovek S et al. Systemic
mastocytosis. In: WHO Classification of Tumours Editorial Board. Haematolymphoid tumours [Internet; beta
version
ahead of print]. International Agency for Research on Cancer;
2023. (WHO classification of tumours series, 5th ed; vol 11). Accessed September 28, 2023.
https://tumourclassification.iarc.who.int/chapters/63
13. Alvarez-Twose I et al. J Allergy Clin Immunol. 2010;125(6):1269-1278.e2.
14. Pieri L et al. Am J Hematol. 2016;91(7):692-699. 15.
González de Olano D et al. J Allergy Clin Immunol. 2008;121(2):519-526. 16.
Gotlib J. Am J Hematol. 2017;92(11):1243-1259. 17. Pardanani A et al.
Leukemia. 2013;27(10):2091-2094.
The information presented here outlines general characteristics of each disorder. Presentation of signs and symptoms may vary from patient to patient.
Irritable bowel syndrome (IBS) is a chronic disorder of gut-brain interaction. IBS is
characterized by symptoms of recurrent abdominal pain
and disordered
defecation.1-3
ISM | IBS |
---|---|
DERMATOLOGIC | |
Monomorphic maculopapular lesions, round, brown or red in color arise predominantly on the thigh and trunk. Lesions may spread or spontaneously disappear, which may or may not indicate disease progression5-7 Symptoms such as flushing, pruritus, and positive Darier sign may be observed5,7,8 The Darier sign is an important clinical feature of mastocytosis skin
lesions. It is defined by the development of a Patient permissions and image reprint from Mikkelsen et al. Dermatol Reports. 2014;6(1):5199. doi:10.4081/dr.2014.5199 have been granted under the Creative Commons License 4.0 https://creativecommons.org/licenses/by-nc/4.0/legalcode |
Not observed1,3 |
GASTROINTESTINAL | |
GI involvement is commonly reported in patients with ISM7,9,10 Symptoms may include diarrhea, abdominal cramping, bloating, |
Recurrent abdominal pain, change in stool frequency, diarrhea,
Abdominal bloating and distention are commonly reported symptoms1-3 |
SYSTEMIC | |
Systemic symptoms include fatigue, anaphylaxis, and weight loss7,10-12 Anaphylaxis may occur; patients typically present with hypotensive syncope and without flushing, urticaria, pruritus, and angioedema7,13,14 Episodes of anaphylaxis appear more likely to develop in patients with mastocytosis
compared to the general population; Hymenoptera |
Not observed1,3 |
OTHER | |
Additional symptoms such as cognitive impairment, dizziness, Please note that this list is not inclusive of all symptoms patients with |
Patients with IBS commonly also have anxiety and depression3 |
GI, gastrointestinal; ISM, indolent systemic mastocytosis.
References:
1. Lacy BE et al. Am J Gastroenterol.
2021;116(1):17-44. 2. Lacy BE. Int J Gen Med. 2016;9:7-17. 3.
Saha L. World J Gastroenterol. 2014;20(22):6759-6773. 4. Mikkelsen CS et al.
Dermatol Reports. 2014;6(1):5199.
5. Hartmann K
et al. J Allergy Clin Immunol. 2016;137(1):35-45. 6. Manjaly Thomas ZR,
Hartmann, K. In: Akin C, ed. Mastocytosis. Springer, Cham; 2020:69-91. 7.
Pyatilova P et al. J Allergy Clin Immunol Pract. 2022;10(8):2015-2024. 8.
Hermans MAW et al. Eur J Intern Med. 2016;30:25-30. 9. Hamilton MJ. In: Akin
C, ed. Mastocytosis. Springer, Cham. 2020:115-122. 10. Lim KH et al.
Blood. 2009;113(23):5727-5736.
11. Taylor F et
al. Orphanet J Rare Dis. 2021;16(1):414. 12. Gülen T et al. In: Akin C, ed.
Mastocytosis. Springer, Cham; 2020:141-155. 13. Verstovek S et al. Systemic
mastocytosis. In: WHO Classification of Tumours Editorial Board. Haematolymphoid tumours [Internet; beta
version ahead of print]. International Agency for Research on Cancer; 2023. (WHO classification of
tumours series, 5th ed; vol 11). Accessed September 28, 2023.
https://tumourclassification.iarc.who.int/chapters/63 14. Alvarez-Twose I et al. J
Allergy Clin Immunol. 2010;125(6):1269-1278.e2. 15. Pieri L et al. Am J
Hematol. 2016;91(7):692-699. 16. González de Olano D et al. J Allergy Clin
Immunol. 2008;121(2):519-526. 17. Pardanani A et al. Leukemia.
2013;27(10):2091-2094.
The information presented here outlines general characteristics of each disorder. Presentation of signs and symptoms may vary from patient to patient.
Inflammatory bowel disease (IBD) is a broad term that includes conditions characterized
by chronic inflammation of the GI tract. IBD
includes Crohn disease and
ulcerative colitis.1
ISM | IBD |
---|---|
DERMATOLOGIC | |
Monomorphic maculopapular lesions, round, brown or red in color arise predominantly on the thigh and trunk. Lesions may spread or spontaneously disappear, which may or may not indicate disease progression3-5 Symptoms such as flushing, pruritus, and positive Darier sign may be observed3,5,6 The Darier sign is an important clinical feature of mastocytosis skin
lesions. It is defined by the development of a Patient permissions and image reprint from Mikkelsen et al. Dermatol Reports. 2014;6(1):5199. doi:10.4081/dr.2014.5199 have been granted under the Creative Commons License 4.0 https://creativecommons.org/licenses/by-nc/4.0/legalcode |
Psoriasis and mucocutaneous lesions associated with IBD can occur7 |
GASTROINTESTINAL | |
GI involvement is commonly reported in patients with ISM5,8,9 Symptoms may include diarrhea, abdominal cramping, bloating, |
Pain in the lower abdomen, swelling, thickening of the bowel wall, |
SYSTEMIC | |
Systemic symptoms include fatigue, anaphylaxis, and weight loss5,9,10,12 Anaphylaxis may occur; patients typically present with hypotensive syncope and without flushing, urticaria, pruritus, and angioedema5,13,14 Episodes of anaphylaxis appear more likely to develop in patients with mastocytosis compared to the general population; Hymenoptera stings are a common trigger5,15,16 |
Weakness, fatigue, and weight changes may be reported1,11 |
OTHER | |
Additional symptoms such as cognitive impairment, dizziness, Please note that this list is not inclusive of all symptoms patients with |
Nutritional deficiencies, anemia, and thromboembolic disease |
GI, gastrointestinal; ISM, indolent systemic mastocytosis.
References:
1. Okobi OE et al. Cureus. 2021;13(8):e16859.
2. Mikkelsen CS et al. Dermatol Reports. 2014;6(1):5199. 3.
Hartmann K et al. J Allergy Clin Immunol. 2016;137(1):35-45. 4. Manjaly Thomas
ZR, Hartmann, K. In: Akin C, ed. Mastocytosis. Springer, Cham; 2020:69-91. 5.
Pyatilova P et al. J Allergy Clin Immunol Pract. 2022;10(8):2015-2024. 6.
Hermans MAW et al. Eur J Intern Med. 2016;30:25-30. 7. Antonelli E et al.
J Clin Med. 2021;10(2):364. 8. Hamilton MJ. In: Akin E, ed.
Mastocytosis. Springer, Cham; 2020:115-122. 9. Lim KH et al. Blood.
2009;113(23):5727-5736. 10. Taylor F et al. Orphanet J Rare Dis.
2021;16(1):414.
11. Fakhoury M et al. J Inflamm
Res. 2014;7:113-120. 12. Gülen T et al. In: Akin C, ed. Mastocytosis.
Springer, Cham; 2020:141-155. 13. Verstovek S et al. Systemic mastocytosis. In: WHO
Classification of Tumours Editorial Board. Haematolymphoid tumours [Internet; beta version ahead of
print]. International Agency for Research on Cancer; 2023. (WHO classification of tumours series, 5th
ed; vol 11). Accessed September 28, 2023. https://tumourclassification.iarc.who.int/chapters/63
14. Alvarez-Twose I et al. J Allergy Clin Immunol. 2010;125(6):1269-1278.e2.
15. Pieri L et al. Am J Hematol. 2016;91(7):692-699.
16. González de Olano D et al. J Allergy Clin
Immunol. 2008;121(2):519-526. 17. Pardanani A et al. Leukemia.
2013;27(10):2091-2094. 18. D'Incà R et al. Diagnostics (Basel).
2023;13(18):2931.
The information presented here outlines general characteristics of each disorder. Presentation of signs and symptoms may vary from patient to patient.
Postural orthostatic tachycardia syndrome (POTS) is a chronic multisystem disorder with
orthostatic tachycardia as its cardinal feature.1,2 It
is also
characterized by frequent symptoms of orthostatic intolerance that occur with standing, an increase in
heart rate ≥30 beats per
minute (or ≥40 beats/min for individuals aged 12-19
years old) when moving from recumbent to standing position, and the absence of
orthostatic hypotension (>20/10 mm Hg drop in blood pressure).1,2
ISM | POTS |
---|---|
DERMATOLOGIC | |
Monomorphic maculopapular lesions, round, brown or red in color arise predominantly on the thigh and trunk. Lesions may spread or spontaneously disappear, which may or may not indicate disease progression4-6 Symptoms such as flushing, pruritus, and positive Darier sign may be observed4.6,7 The Darier sign is an important clinical feature of mastocytosis skin
lesions. It is defined by the development of a Patient permissions and image reprint from Mikkelsen et al. Dermatol Reports. 2014;6(1):5199. doi:10.4081/dr.2014.5199 have been granted under the Creative Commons License 4.0 https://creativecommons.org/licenses/by-nc/4.0/legalcode |
Some patients may experience episodes of flushing and urticaria1 |
GASTROINTESTINAL | |
GI involvement is commonly reported in patients with ISM6,8,9 Symptoms may include diarrhea, abdominal cramping, bloating, |
Nausea, bloating, diarrhea, abdominal pain, and vomiting may occur1,2 |
SYSTEMIC | |
Systemic symptoms include fatigue, anaphylaxis, and weight loss6,9-11 Anaphylaxis may occur; patients typically present with hypotensive syncope and without flushing, urticaria, pruritus, and angioedema6,12,13 Episodes of anaphylaxis appear more likely to develop in patients with mastocytosis compared to the general population; Hymenoptera stings are a common trigger6,14,15 |
Symptoms vary between individuals and can include generalized |
OTHER | |
Additional symptoms such as cognitive impairment, dizziness, Please note that this list is not inclusive of all symptoms patients with |
Some patients with POTS have comorbid conditions or symptoms Other common signs and symptoms are related to orthostatic Sleep disturbances, anxiety, tachycardia, tremor, angina-like chest pain, |
GI, gastrointestinal; ISM, indolent systemic mastocytosis.
References:
1. Raj SR et al. CMAJ. 2022;194(10):e378-e385.
2. Sheldon RS et al. Heart Rhythm. 2015;12(6):e41-e63. 3.
Mikkelsen CS et al. Dermatol Reports. 2014;6(1):5199. 4. Hartmann K et al.
J Allergy Clin Immunol. 2016;137(1):35-45. 5. Manjaly Thomas ZR, Hartmann, K.
In: Akin C, ed. Mastocytosis. Springer, Cham; 2020:69-91. 6. Pyatilova P et
al. J Allergy Clin Immunol Pract. 2022;10(8):2015-2024. 7. Hermans MAW et al.
Eur J Intern Med. 2016;30:25-30. 8. Hamilton MJ. In: Akin C, ed.
Mastocytosis. Springer, Cham. 2020:115-122. 9. Lim KH et al. Blood.
2009;113(23):5727-5736. 10. Taylor F et al. Orphanet J Rare Dis.
2021;16(1):414.
11. Gülen T et al. In: Akin C, ed.
Mastocytosis. Springer, Cham; 2020:141-155. 12. Verstovek S et al. Systemic
mastocytosis. In: WHO Classification of Tumours Editorial Board. Haematolymphoid tumours [Internet;
beta version ahead of print]. International Agency for Research on Cancer;
2023. (WHO classification of tumours series, 5th ed; vol 11). Accessed September 28, 2023.
https://tumourclassification.iarc.who.int/chapters/63 13. Alvarez-Twose I et al. J Allergy Clin Immunol.
2010;125(6):1269-1278.e2. 14. Pieri L et al. Am J Hematol. 2016;91(7):692-699.
15. González de Olano D et al. J Allergy Clin Immunol. 2008;121(2):519-526.
16. Pardanani A et al. Leukemia. 2013;27(10):2091-2094.
The information presented here outlines general characteristics of each disorder. Presentation of signs and symptoms may vary from patient to patient.
Idiopathic anaphylaxis (IA) is a multisystem disorder, which presents clinically as
anaphylaxis of unknown etiology. IA is a diagnosis of
exclusion since its
triggers cannot be identified despite a detailed history and careful diagnostic assessment.1,2
ISM | IA |
---|---|
DERMATOLOGIC | |
Monomorphic maculopapular lesions, round, brown or red in color arise predominantly on the thigh and trunk. Lesions may spread or spontaneously disappear, which may or may not indicate disease progression4-6 Symptoms such as flushing, pruritus, and positive Darier sign may be observed4,6,7 The Darier sign is an important clinical feature of mastocytosis skin
lesions. It is defined by the development of a Patient permissions and image reprint from Mikkelsen et al. Dermatol Reports. 2014;6(1):5199. doi:10.4081/dr.2014.5199 have been granted under the Creative Commons License 4.0 https://creativecommons.org/licenses/by-nc/4.0/legalcode |
Acute onset of an illness (minutes to several hours) with simultaneous involvement of the skin, mucosal tissue, or both (eg, generalized hives, pruritus or flushing, swollen lips-tongue-uvula)1,8 |
GASTROINTESTINAL | |
GI involvement is commonly reported in patients with ISM6,9,10 Symptoms may include diarrhea, abdominal cramping, |
Severe abdominal symptoms such as diarrhea, repetitive vomiting, |
SYSTEMIC | |
Systemic symptoms include fatigue, anaphylaxis, and weight loss6,10-12 Anaphylaxis may occur; patients typically present with hypotensive syncope and without flushing, urticaria, pruritus, and angioedema6,13,14 Episodes of anaphylaxis appear more likely to develop in patients with mastocytosis compared to the general population; Hymenoptera stings are a common trigger6,15,16 |
Systemic symptoms include episodes of anaphylaxis, which may |
OTHER | |
Additional symptoms such as cognitive impairment, dizziness, Please note that this list is not inclusive of all symptoms patients with |
Symptoms of respiratory and/or cardiovascular compromise such as Reduced blood pressure and associated symptoms of end-organ |
This section provides some information that may be helpful in further identifying patients with ISM vs IA and is not inclusive of all distinguishing features. Please refer to the formal guidelines for each disorder, if available, for a more comprehensive list.
Patients with IA may be diagnosed with SM upon further evaluation. Therefore, patients with recurrent, unexplained episodes of anaphylaxis should be screened further for the presence of mastocytosis.18-20
GI, gastrointestinal; ISM, indolent systemic mastocytosis; SM, systemic mastocytosis.
References:
1. Nwaru BI et al. Curr Treat Options Allergy.
2017;4(3):312-319. 2. Sampson HA et al. J Allergy Clin Immunol.
2005;115(3):584-591. 3. Mikkelsen CS et al. Dermatol Reports. 2014;6(1):5199.
4. Hartmann K et al.
J Allergy Clin Immunol.
2016;137(1):35-45. 5. Manjaly Thomas ZR, Hartmann, K. In: Akin C, ed.
Mastocytosis. Springer, Cham; 2020:69-91. 6. Pyatilova P et al. J Allergy
Clin Immunol Pract. 2022;10(8):2015-2024.
7.
Hermans MAW et al. Eur J Intern Med. 2016;30:25-30. 8. Cardona V et al.
World Allergy Organ J. 2020;13(10):100472. 9. Hamilton MJ. In: Akin E, ed.
Mastocytosis. Springer, Cham; 2020:115-122. 10. Lim KH et al.
Blood. 2009;113(23):5727-5736. 11. Taylor F et
al. Orphanet J Rare Dis. 2021;16(1):414. 12. Gülen T et al. In: Akin C, ed.
Mastocytosis. Springer, Cham; 2020:141-155. 13. Verstovek S et al. Systemic
mastocytosis. In: WHO Classification of Tumours Editorial Board. Haematolymphoid tumours [Internet; beta
version ahead of print]. International Agency for Research on Cancer; 2023. (WHO classification of
tumours series, 5th ed; vol 11). Accessed September 28, 2023.
https://tumourclassification.iarc.who.int/chapters/63 14. Alvarez-Twose I et al. J
Allergy Clin Immunol. 2010;125(6):1269-1278.e2. 15. Pieri L et al. Am J
Hematol. 2016;91(7):692-699. 16. González de Olano D et al. J Allergy Clin
Immunol. 2008;121(2):519-526. 17. Pardanani A et al. Leukemia.
2013;27(10):2091-2094. 18. Pardanani A. Am J Hematol. 2023;98(7):1097-1116.
19. Carter MC et al. J Allergy Clin Immunol.
2018;141(1):180-188.e3. 20. American Academy of Allergy, Asthma, and Immunology and
American College of Allergy, Asthma, and Immunology. Anaphylaxis: a 2023 practice parameter update,
2023. Accessed September 29, 2023.
https://college.acaai.org/wp-content/uploads/2023/04/Anaphylaxis-2023-Practice-Parameter-Update-01APR2023.pdf